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Hydrocephalus is perhaps one of the most unsettling conditions to affect infants. The baby develops massive swelling of the brain due to the retention of surplus cerebrospinal fluid. And, the disconcerting swelling holds the potential to damage the brain eventually. The only treatment for hydrocephalus in infants is surgery followed by regular check-ups. Most parents have limited information on the condition and the fundamental reasons behind it. MomJunction helps you understand hydrocephalus, its symptoms, prognosis, and treatment.

What Is Hydrocephalus In Babies?

Hydrocephalus is a condition where excess cerebrospinal fluid remains in the cavities of the brain, causing the brain and skull to swell. Cerebrospinal fluid, or CSF, is a clear liquid that circulates within the nervous system to provide nourishment and cushion the brain and spinal cord (1).

The fluid is produced by a part of a brain called the choroid plexus from where it moves into a series of channels within the brain called ventricles (2). From the ventricles, the fluid circulates to parts of the nervous system.

The ventricles act as a center for transfer of any surplus CSF into the bloodstream. When ventricles fail to drain the fluid, it accumulates in the brain, causing the brain and the skull to swell. The accumulation of excess CSF leads to swelling. But why does the excess CSF accumulate? Let’s find out.

What Causes Hydrocephalus In Babies?

The fundamental reason behind the accumulation of cerebrospinal fluid depends on the type of hydrocephalus – congenital hydrocephalus or acquired hydrocephalus. Here are the reasons behind both the conditions.

i) Causes of congenital hydrocephalus:

This condition occurs at the time of the birth, due to the reasons listed below:

Ventriculomegaly: It is a condition where the ventricles of the brain become larger than normal due to a congenital disability. Large ventricles cause irregularity in the flow of CSF leading to hydrocephalus.

Arachnoid cyst: These cysts are small, abnormal pockets of cerebrospinal fluid contained within the arachnoid layer, which is among the several membranes covering the brain (3). Arachnoid cysts are self-contained but are still connected to the ventricles thus affecting the pressure of CSF on the brain.

Spina bifida: It is a birth defect where the bones of the spine do not fuse properly. It causes the spinal cord and the rest of the nervous system to form abnormally. Excess CSF is among the anomalies that are a result of spina bifida (4).

Maternal infections during pregnancy: Severe infections to the mother during pregnancy increase the risk of a baby being born with hydrocephalus. Diseases such as rubella and mumps affecting the mother have been linked to the birth of infants with congenital hydrocephalus (5).

The above reasons are a result of genetic defects and the unborn baby displays the first signs of congenital hydrocephalus in the womb.

ii) Causes of acquired hydrocephalus:

Acquired hydrocephalus happens after birth and is not caused congenitally. In this case, a newborn would be healthy at birth but would develop hydrocephalus later due to any of the below-mentioned reasons:

Intraventricular hemorrhage: Bleeding happens within the brain causing the blood to flow into the ventricles, mix with the CSF, and increase the fluid pressure. This condition mostly occurs in premature babies that have underdeveloped and immensely delicate blood vessels that rupture randomly inside the brain for no definite reason. Intraventricular hemorrhage and the consequential hydrocephalus are rare in full-term infants (6).

Head injuries: Injuries to the head can cause bleeding within ventricles leading to hydrocephalus. The bleeding is similar to the one resulting from intraventricular hemorrhage.

Infections: Diseases affecting the nervous system can lead to hydrocephalus. One example is meningitis, an inflammation of the protective membrane surrounding the brain due to an infection by a pathogen. Meningitis interferes with the reabsorption of CSF, thus causing hydrocephalus (7).

Brain cyst or tumor: Cyst and tumors can obstruct the ventricles and reduce their volume, which eventually causes excess CSF to stay in the brain. This type of hydrocephalus caused by blockage is called non-communicating hydrocephalus (8).

Poor absorption of CSF: The CSF can flow through the ventricles unobstructed, but the bloodstream is unable to absorb any excess fluid due to the defects in the ventricle. The swelling of the brain, in this case, is called communicating hydrocephalus.

Acquired hydrocephalus can occur anytime during a baby’s infanthood. There is a condition though, which sounds quite similar to hydrocephalus, but is different.

Benign external hydrocephalus – is it hydrocephalus?

Benign external hydrocephalus, also known as external hydrocephalus, is the enlargement of the subarachnoid spaces, which is tissue-filled space around the brain. The engorgement of the arachnoid spaces causes the brain to swell and the skull to expand. However, there is little to no increase in the ventricle size. Also, there is no impact on the flow of the CSF. The condition is self-limiting, resolves within a few years, and seldom causes complications unlike true hydrocephalus (9). Infants with the condition do not show any adverse symptoms, and no treatment is required (10). Therefore the disorder is different from hydrocephalus and should not be confused with it.

You can avoid this confusion when you are able to identify the ‘real’ hydrocephalus in babies.

What Are The Symptoms Of Hydrocephalus In Babies?

A baby suffering from hydrocephalus will show the following signs of the condition (11):

Unusual swelling of the head: It is the first and earliest sign of hydrocephalus in infants. The top of the skull would appear to increase in diameter with every passing day. The skull would expand, and a bulging, tense, soft spot can be felt right at the top of the head. The swelling makes the baby’s head seem odd and unusually large in proportion to the body. Even the mildest cases of hydrocephalus result in the rapid expansion of skull.

Splitting of skull bones: Parents may spot seams appearing at the different parts of the head. These seams are sutures, the junction of the bones of the skull that lies underneath the skin. Rapid swelling of the brain causes the skull bones to expand leading to the widening of the sutures.

Drooping of the eyes: The eyes have a permanent downward cast and it appears that the baby always looks down without moving the eyes much. It is also called sunsetting of the eyes.

Poor feeding, and vomiting: The baby will stop feeding properly once the swelling emerges. Vomiting becomes common and leads to poor appetite.

How Is Hydrocephalus In Babies Diagnosed?

Physical examination: The doctor measures the circumference of the infant’s head and checks for abnormal growth. Eyes are inspected to determine if they are sunken and if the baby has problems moving them. The soft spot on a baby’s head, called fontanel, is checked for any swelling.

Ultrasound examination: The doctor places an ultrasound probe on top of the head. The high-frequency ultrasound waves form an image of the brain that is assessed to observe any accumulation of cerebrospinal fluid.

Computerized tomography (CT): Computerized tomography, also known as CT scan, uses multiple X-ray images to create a three-dimensional picture of the brain. It helps the doctor spot the precise location of the swelling. CT scan takes about 20 minutes and requires the baby to stay still. For this reason, the doctor may give a mild sedative to the infant before taking the X-ray.

Magnetic resonance imaging (MRI): The test uses magnetic field and radio waves to create a virtual, three-dimensional image of the brain. The digital image gives an accurate representation of every part of the brain. MRI process requires staying still, and can take up to an hour depending on the type of equipment. Some hospitals are equipped with fast-scanning MRI machines that take only five minutes and do not require sedation.

The above methods help diagnose hydrocephalus in an infant after they are born. But the condition can be diagnosed in an unborn baby as well.

Diagnosing hydrocephalus in the womb:

A mother can get the fetus screened for hydrocephalus during her regular pregnancy checkup. The following steps are used to diagnose hydrocephalus in an unborn baby:

Ultrasound scan: Doctors will check the size of the ventricles during routine ultrasound tests. They will assess and comment on the brain development of the growing fetus.

Amniocentesis: If a doctor confirms hydrocephalus in the fetus, a second assessment is made through the process called amniocentesis. Here, a needle is inserted through the mother’s belly straight into the amniotic sac to extract a sample of the amniotic fluid. The fluid is then screened for genetic mutations that can help determine disorders such as ventriculomegaly that can lead to hydrocephalus (13).

Diagnosis before birth helps the parents and doctors to be well-prepared for treatment once the baby is born.

How Is Hydrocephalus In Babies Treated?

The disorder is treatable only through surgery and no drugs can cure the condition. The following surgical interventions are used for the treatment (14):

Shunt surgery: It is the most common treatment method for hydrocephalus in infants. The shunt treatment procedure uses two components – shunt and valve. The shunt is a long tube, like a catheter, made from silicone. An opening of the tube is placed inside the ventricle where excess CSF is accumulated. The tube is then routed under the skin to end at a heart chamber or stomach, for reabsorption of the fluid. A valve is also attached to the shunt at a point near the ventricle. The one-way valve controls the flow and pressure of CSF draining out of the ventricles. It prevents back-flow of CSF into the ventricle even when the patient changes position, and mitigates excess drainage of cerebrospinal fluid.

Endoscopic third ventriculostomy: The surgeon makes an incision into the ventricle and inserts a probe with a camera. A spot on the ventricle is identified, and a hole is drilled to let the fluid drain out into the brain, from where blood vessels absorb it. This method is less painful and invasive yet less efficient for infants unless modified with an accompanying procedure.

Endoscopic third ventriculostomy with choroid plexus coagulation: A regular ventriculostomy operation is followed by destroying a segment of choroid plexus, which is a part of the brain that produces cerebrospinal fluid. An electric impulse is used to null the functions of choroid plexus thus preventing it from producing excess cerebrospinal fluid. Endoscopic third ventriculostomy with choroid plexus coagulation is the only method of treating hydrocephalus in premature infants and those suffering from it due to spina bifida.

Do note that none of the above procedures are free of complications, and hence, must be done by professionals.

What Are The Complications And Limitations Of The Treatment?

Before opting for the treatment for hydrocephalus, you need to know of the complications associated with it.

Hydrocephalus can recur: Surgical procedures cannot stop the production of cerebrospinal fluid (15). Therefore, there is a risk of relapse. Nevertheless, surgical procedures successfully cut down the symptoms and let the infant manage hydrocephalus comfortably for the rest of the life.

Surgical procedures have limitations: A ventriculostomy procedure works only if the swelling is caused by blockage of ventricles. For other reasons, shunt procedure is the only choice. Shunt surgery requires elaborate preparations such as making the baby fast for six hours before the surgery. After the operation, the baby needs to lay in bed and not move for 24 hours. The total hospital stay can last for up to three days. All this can be overwhelming and painful for the little one.

Long-term follow-up is mandatory: Shunts need to be inspected and changed periodically. The performance of the valve has to be monitored at set intervals by the doctor. An average shunt can last for five years, but the replacement interval can vary depending on the severity of the condition and the doctor’s recommendations.

High chances of complications: Shunt valves are prone to mechanical failures, overtraining, underdraining, and blockage. Bacterial infections after surgery are quite likely to happen within the first three months. There is 30% chance of a shunt failure in the first year after surgery. Even a minor infection or problem makes it imperative to replace the entire shunt system. Moreover, ventriculostomy can cause bleeding inside the brain, leading to severe pain.

Spotting infections after surgery:

The baby will show the following symptoms if he develops an infection after surgery.

Bulging or sinking of the head area where the shunt enters the brain

Swelling or redness on the body part where the shunt tube passes

Vomiting

Poor feeding

Irritability and frequent crying

Drowsiness and lethargy

Abdominal pain

The baby will show a recurrence of all the symptoms of hydrocephalus. If you suspect of infection, take your baby to a doctor with no second thoughts.

Once the baby is home after surgery, you must indulge in some home care.

How To Care For The Baby After Surgery?

Surgery is a big thing for a baby. They can’t express their pain and discomfort, and therefore get frustrated. Parents need to take utmost care of the baby after the hydrocephalus-correction surgery.

Feed the baby the usual way. The baby would have stayed empty stomach for the surgery and would be famished. Once the little one regains consciousness, you can resume breastfeeding right at the hospital after getting the doctor’s approval. Normal breastfeeding can be continued at home since shunt in the stomach will not interfere with digestion.

Give solid food as well. Experts recommend giving adequate fruits and vegetables to the baby if he can consume solid food (16). Solid diet will provide sufficient micronutrients and vitamins to the baby, helping in the healing process.

Do not bathe the baby till stitches are removed. The doctor may use dissolvable stitches or stitches that are removed later. Do not bathe the baby till the incision has been cleared of the stitches. Removal of the stitches means the skin has closed and is safe to come in contact with water and substances such as soap.

Keep the incision area clean and dry. Use warm, soapy water to dress the spot regularly and ensure that the wound is dry. Learn about shunt care and hygiene from the doctor before leaving the hospital.

No strenuous physical activities for some time. It takes time for the surgery wounds to heal and the shunt to settle in its place. Therefore, a baby should stay away from potentially exerting games and activities for six weeks after surgery (17). The baby can go back to daycare after seven days, but should not indulge in anything strenuous.

Train the infant not to touch the shunt. Once the baby has recovered from the surgery, he will be conscious and curious about the strange bump on his head. Older infants are quite likely to play or poke at the bump, which is the shunt entrance into the brain. Teach your baby to not touch the shunt by discouraging him with words like “No” every time they do it. Keep the baby’s hand occupied with a toy every time you sense he is bored and about to touch the shunt to play.

The condition can be easily managed with the help and guidance of the doctor. If you are worried about the future of the baby with hydrocephalus, then MomJunction can help.

Prognosis Of Hydrocephalus In Infants

Here are some important points you need to know:

Infants with hydrocephalus have good survival rate with about 95% of infants surviving to live a long, healthy life. The life expectancy is at par with normal babies (18). An excellent survival rate is possible through treatment, which may be complicated and imperfect, but still, gives the baby a better chance at life than living with hydrocephalus.

The effect depends on the severity of hydrocephalus, and that influences the outcome of any long-term complications (19). Babies with mild hydrocephalus may recover better than those with severe hydrocephalus. However, both would require frequent screenings for any delay in growth.

Irreversible brain damage is possible in congenital hydrocephalus. This results in parts of the brain failing to perform regular functions (20). It can lead to other neurological problems such as epilepsy and seizures.

There is a high probability of a baby with hydrocephalus suffering from developmental delay (21). Problems include learning disabilities, impaired speech, short attention span, memory issues, and vision problems. Nevertheless, experts state that about 50% of babies with hydrocephalus grow up to have normal cognitive functions and intelligence.

Rehabilitation and educational therapies can help a baby make up for the lost development. The National Institute of Neurological Disorders and Stroke (NINDS), US, states that rehabilitation therapies help an infant with hydrocephalus lead a normal life with minimal limitations. Therapists specializing in hydrocephalus can help mitigate the chances of any developmental issues.

Hydrocephalus need not hinder a baby’s normal growth, but given a chance, any parent would, no doubt, want to avoid it. Alas, that could happen.

Can Hydrocephalus In Babies Be Prevented?

Unfortunately, there is no way to prevent hydrocephalus despite early detection in the womb (22). Researchers are still working to find a way to prevent and cure the condition.

But parents can work on mitigating the risk:

Get regular checkups during pregnancy. Never miss a doctor’s appointment when you are pregnant, and stick to the schedule of ultrasounds. An early detection provides the best chance for a baby to survive and live a normal life after birth.

Get immunized during pregnancy. Consult the doctor and get yourself vaccinated against diseases such as meningitis as the disease may impact the brain development of the fetus. Protect yourself from general illness to minimize the risk of any adverse effects on fetal development.

Protect infants against head injury. Baby proof your house and secure objects that have a potential to come in the way of a crawling infant. Use a crib with guard rails or bars to prevent a baby from falling. When traveling by car, use baby safety seat.

Immunize the baby. Protecting the baby against illnesses could minimize the risk of their effects on the brain. Consult the baby’s pediatrician for a schedule of vaccinations and follow it.

Hydrocephalus may be a life-long condition, but most infants have sailed through it comfortably. Remember to stay alert about any complications after treatment and take the baby for regular check-ups. Hydrocephalus is certainly complex, but timely intervention through treatment is the key to ensure a long and healthy life for the baby.

Have any tips to share with us? Leave them in the comments section below.

Rohit Garoo

Rohit Garoo took writing as a profession right after finishing his MBA in Marketing. Earlier he graduated with a Bachelor’s degree in Botany & Zoology from the autonomous St. Xavier’s College in Mumbai. Rohit has also done a Stanford University certification course on breastfeeding. This botanist-zoologist turned writer excels at life sciences, and at MomJunction he writes everything about pediatrics and maternal care. In between writing and being overly curious, he spends time cooking, reading, and playing video games. LinkedIn profile – linkedin.com/in/rohit-garoo-263115aa

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